Lupus nephritis and neuropsychiatric abnormalities are two of the most severe manifestations of childhood-onset systemic lupus erythematosus. And while a familiar constellation of laboratory and biopsy results can identify kidney involvement and damage, neuropsychiatric symptoms can be more difficult to pinpoint.
“Compared to lupus nephritis in children, there hasn’t been the same focus on neuropsychiatric lupus historically because it presents with so many varied manifestations,” said Ekemini Ogbu, MD, MSc, FAAP, Assistant Professor of Pediatrics, University of Cincinnati/Cincinnati Children’s Hospital Medical Center, and Interim Director, Cincinnati Children’s Hospital Multiple Sclerosis and Neuroimmunology Center. “Trying to study nervous system involvement has been challenging, and the measures we collect to assess lupus disease activity do not always correlate with what the patient or parents are reporting. New research is making this an expanding field.”
Dr. Ogbu will moderate Neuropsychiatric Manifestations in Childhood Systemic Lupus Erythematosus on Saturday, November 12, from 5–6 p.m. ET, in Terrace Ballroom I of the Pennsylvania Convention Center. Meeting participants have the option to attend the session in person or on the meeting website via livestream, or to view the session on demand.
Mood disorders and cognitive changes are among the most common neuropsychiatric manifestations of lupus in children and adolescents, as well as the most challenging. One challenge in the care of children with neuropsychiatric lupus is who should take the lead in screening and management: primary care pediatricians or pediatric rheumatologists.
“There are specific guidelines for pediatricians to screen children with tools that have been validated in the general population,” said Tamar Rubinstein, MD, MS, Assistant Professor in Pediatrics and in Psychiatry and Behavioral Sciences and Pediatric Rheumatology, Children’s Hospital at Montefiore. “Some pediatric rheumatologists may believe we should leave it in the domain of pediatricians. We already have a full plate when it comes to all the other things we need to consider as rheumatologists.”
However, there is a compelling argument for the involvement of rheumatologists.
“Neuropsychiatric symptoms are highly prevalent in our patients, especially those with lupus, and they affect lupus patients in very special ways,” Dr. Rubinstein continued. “We have to consider mental health as part of their overall disease management.”
There are similar arguments around cognitive disorders associated with pediatric lupus.
“One of the very common manifestations of neuropsychiatric lupus is change in cognition,” said Hermine I. Brunner, MD, MSc, MBA, Professor of Pediatrics and of Rheumatology, Cincinnati Children’s Hospital, and Chair of the Pediatric Rheumatology Collaborative Study Group. “And we don’t have a lab test like we do for strep throat, which always instills doubts.”
What looks like depression or cognitive problems in an adolescent may just be a teenager having bad day. It could also be caused by lupus. Or it may be a combination of the two.
“Given that children with lupus are most closely followed by rheumatologists, it’s up to us to diagnose and treat neuropsychiatric lupus in children,” Dr. Brunner said.
There is also the argument that pediatricians prefer to steer clear of lupus patients because of the complex treatment regimens involved.
“Pediatricians are very careful in rendering care to children with lupus, even for their acute health problems, because they fear drug-drug interactions or secondary exacerbation of lupus because of a certain medication or treatment approach that is outside their control,” Dr. Brunner said. “We need to treat the whole patient.”
Neuropsychiatric disorders are particularly difficult to diagnose and treat in lupus due to the lack of reliable biomarkers and laboratory testing. New advances in imaging appear to be filling that gap.
“Too often you have nonspecific findings that are not directly related to the manifestation,” explained Simone Appenzeller, MD, Associate Professor of Orthopedics, Rheumatology and Traumatology, University of Campinas School of Medicine, São Paulo, Brazil. “We now have microstructural abnormalities that appear on imaging before symptoms worsen, especially in cognitive function. These changes may be the cause of more cognitive dysfunction and more difficulties adapting to daily living, getting jobs, and keeping jobs.”